We studied damage accrual and factors determining development and progression of damage in an international cohort of systemic lupus erythematosus (SLE) patients.
The Systemic Lupus International ...Collaborating Clinics (SLICC) Inception Cohort recruited patients within 15 months of developing four or more 1997 American College of Rheumatology (ACR) criteria for SLE; the SLICC/ACR damage index (SDI) was measured annually. We assessed relative rates of transition using maximum likelihood estimation in a multistate model. The Kaplan-Meier method estimated the probabilities for time to first increase in SDI score and Cox regression analysis was used to assess mortality.
We recruited 1722 patients; mean (SD) age 35.0 (13.4) years at cohort entry. Patients with damage at enrolment were more likely to have further worsening of SDI (SDI 0 vs ≥1; p<0.001). Age, USA African race/ethnicity, SLEDAI-2K score, steroid use and hypertension were associated with transition from no damage to damage, and increase(s) in pre-existing damage. Male gender (relative transition rates (95% CI) 1.48 (1.06 to 2.08)) and USA Caucasian race/ethnicity (1.63 (1.08 to 2.47)) were associated with SDI 0 to ≥1 transitions; Asian race/ethnicity patients had lower rates of new damage (0.60 (0.39 to 0.93)). Antimalarial use was associated with lower rates of increases in pre-existing damage (0.63 (0.44 to 0.89)). Damage was associated with future mortality (HR (95% CI) 1.46 (1.18 to 1.81) per SDI point).
Damage in SLE predicts future damage accrual and mortality. We identified several potentially modifiable risk factors for damage accrual; an integrated strategy to address these may improve long-term outcomes.
The Lupus Foundation of America (LFA) convened an international working group to obtain a consensus definition of disease flare in lupus. With help from the Paediatric Rheumatology International ...Trials Organization (PRINTO), two web-based Delphi surveys of physicians were conducted. Subsequently, the LFA held a second consensus conference followed by a third Delphi survey to reach a community-wide agreement for flare definition. Sixty-nine of the 120 (57.5%) polled physicians responded to the first survey. Fifty-nine of the responses were available to draft 12 preliminary statements, which were circulated in the second survey. Eighty-seven of 118 (74%) physicians completed the second survey, with an agreement of 70% for 9/12 (75%) statements. During the second conference, three alternative flare definitions were consolidated and sent back to the international community. One hundred and sixteen of 146 (79.5%) responded, with agreement by 71/116 (61%) for the following definition: “A flare is a measurable increase in disease activity in one or more organ systems involving new or worse clinical signs and symptoms and/or laboratory measurements. It must be considered clinically significant by the assessor and usually there would be at least consideration of a change or an increase in treatment.” The LFA proposes this definition for lupus flare on the basis of its high face validity.
PurposeDiagnosis and treatment of systemic lupus erythematosus (SLE) are based on the compilation ofcomplex sets of clinical data, often established over several years. Reading through those large ...matrices canbe time-consuming and require specifi c training. Thus, our aim was to create a visual representation thatmakes it easier to access the global health status of an SLE patient, and to use it as a knowledge transfertool.MethodsFirst, we selected clinical criteria that are representative, useful, and revealing of the medicalsituation in SLE. Using R language programming, we developed a script that automatically transposesclinical data into an attractive image, whose graphical characteristics refl ect the selected clinical criteria.ResultsThe visual representation is a butterfl y, the emblematic symbol of lupus, which incorporates shadesof purple, the color of lupus awareness, and is compliant for people with color blindness. The visualgraphically provides eleven key clinical criteria, including patient-reported outcomes: age, sex, diseaseactivity (SLE Disease Activity Index 2000), organ damage (Systemic Lupus International Collaborating ClinicsDamage Index), comorbidities (Charlson Comorbidity Index), physical- and mental-health-related quality oflife (component summary scores from the 36-Item Short Form Survey), medication with antimalarial drugs,immunosuppressants, biologics and dosages of prednisone.Abstract PO.6.125 Figure 1Evolution of the health status of an SLE patient over years through the butterfly toolConclusionsWe implemented an automated tool that transposes complex and heterogeneous clinical dataobtained from patients with SLE, into an intuitive visual medium. In addition to helping physicians to rapidlycomprehend the health status of SLE patients, this data visualization shall facilitate communication betweenphysicians, scientists, patients, and the public in general. Also, we believe it could help patients takeownership of their own condition, raise public awareness about SLE, and act as an incentive to furtherinvolve patients in research.
IntroductionLoneliness is prevalent among patients with inflammatory rheumatic diseases (IRDs), however the COVID-19 pandemic may intensify loneliness among patients with IRDs, as they are at higher ...risk of severe illness. Early evidence suggests that this was indeed the case during the early stages; however, it remains largely unknown whether loneliness remains present and what factors are associated. The objective of the present study was to identify risk and protective factors associated with loneliness in individuals living with IRDs in the later stage of the COVID-19 pandemic.MethodsData from an online cross-sectional survey study of individuals with systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) between May 2022 and February 2023. Participants were recruited from a list of patients already enrolled in a multi-site longitudinal observation study. All participants provided informed consent. Loneliness was assessed with the University of California, Los Angeles Loneliness Scale (Version 3), Short Form 3-item (UCLA- LS3-SF3). The Impact of Event Scale-Revised (IES-R) assessed post-traumatic stress symptoms (PTSS) caused by the COVID-19 pandemic. The Patient Health Questionnaire-8 (PHQ-8) measured symptoms of depression. Resilience and concerns related to COVID-19 were also assessed. Descriptive statistics and linear regressions were conducted.ResultsThe study population was n = 160 (SLE = 102, RA = 58), mean age 60.1 years (±13) and 21.9% (n=35) were men. Almost one third (28.1%) reported moderate to severe loneliness. (UCLA-LS3-SF3 score ≥6), with statistically significant difference between both disease groups (SLE = 36.3%; RA= 13.8%). Among participants with SLE, gender (men), psychological burden from the COVID-19 pandemic, and higher depressive symptoms were independently associated with greater loneliness, accounting for 38% of the variance (table 1). Among individuals living with RA, identifying as female and greater psychological burden from the COVID-19 pandemic were independently associated with greater loneliness, accounting for 55% of the variance.DiscussionThe results suggest that special attention to men with SLE and women with RA is needed when targeting loneliness in people with IRDs. More attention to strategies to decrease depressive symptoms and the psychological burden of the pandemic in patients living with IRDs are needed in future public health crises.Abstract 1403 Table 1Factors associated with loneliness during the later stages of the COVID-19 pandemic in people with IRDs Parameter Β1 p-value 95% C.I. Lower Upper SLE Age .013 .877 −.022 .025 Gender − .178 .032 - 2.55 − .199 Psychological Burden of pandemic .212 .035 .002 .063 Covid-19 concerns .062 .514 −.025 .050 Depression .333 .002 .046 .208 Resilience −.154 .136 −.903 .125 RA Age .022 .855 −.029 .035 Gender .229 .032 .051 1.12 Psychological Burden of pandemic .380 .014 .010 .081 Covid-19 concerns .240 .114 −.010 .087 Depression .109 .352 −.046 .127 Resilience −.170 .100 −.707 .063 1Standardized beta coefficients. CI = confidence interval.
Objective
To evaluate the subsequent rate of thrombosis among women with obstetric antiphospholipid syndrome (Ob‐APS) in a multicentre database of antiphospholipid antibody (aPL)‐positive patients, ...and the clinical utility of the adjusted Global Antiphospholipid Syndrome Score (aGAPSS), a validated tool to assess the likelihood of developing new thrombosis, in this group of patients.
Design
Retrospective study.
Setting
The Antiphospholipid Syndrome Alliance for Clinical Trials and International Networking Clinical Database and Repository.
Population
Women with Ob‐APS.
Methods
Comparison of clinical and laboratory characteristics and measurement of aGAPSS in women with Ob‐APS, with or without thrombosis, after initial pregnancy morbidity (PM).
Main outcome measures
Risk factors for thrombosis and aGAPSS.
Results
Of 550 patients, 126 had Ob‐APS; 74/126 (59%) presented with thrombosis, and 47 (63%) of these women developed thrombosis after initial PM, in a mean time of 7.6 ± 8.2 years (4.9/100 patient years). Younger age at diagnosis of Ob‐APS, additional cardiovascular risk factors, superficial vein thrombosis, heart valve disease, and multiple aPL positivity increased the risk of first thrombosis after PM. Women with thrombosis after PM had a higher aGAPSS compared with women with Ob‐APS alone median 11.5 (4–16) versus 9 (4–13); P = 0.0089.
Conclusion
Based on a retrospective analysis of our multicentre aPL database, 63% of women with Ob‐APS developed thrombosis after initial obstetric morbidity; additional thrombosis risk factors, selected clinical manifestations, and high‐risk aPL profile increased the risk. Women with subsequent thrombosis after Ob‐APS had a higher aGAPSS at entry to the registry. We believe that aGAPSS is a valid tool to improve risk stratification in aPL‐positive women.
Tweetable
More than 60% of women with obstetric antiphospholipid syndrome had thrombosis after initial pregnancy morbidity.
Tweetable
More than 60% of women with obstetric antiphospholipid syndrome had thrombosis after initial pregnancy morbidity.
To determine the frequency, accrual, attribution and outcome of neuropsychiatric (NP) events and impact on quality of life over 3 years in a large inception cohort of patients with systemic lupus ...erythematosus (SLE).
The study was conducted by the Systemic Lupus International Collaborating Clinics. Patients were enrolled within 15 months of SLE diagnosis. NP events were identified using the American College of Rheumatology case definitions, and decision rules were derived to determine the proportion of NP disease attributable to SLE. The outcome of NP events was recorded and patient-perceived impact determined by the SF-36.
1206 patients (89.6% female) with a mean (+/-SD) age of 34.5+/-13.2 years were included in the study. The mean disease duration at enrollment was 5.4+/-4.2 months. Over a mean follow-up of 1.9+/-1.2 years, 486/1206 (40.3%) patients had > or =1 NP events, which were attributed to SLE in 13.0-23.6% of patients using two a priori decision rules. The frequency of individual NP events varied from 47.1% (headache) to 0% (myasthenia gravis). The outcome was significantly better for those NP events attributed to SLE, especially if they occurred within 1.5 years of the diagnosis of SLE. Patients with NP events, regardless of attribution, had significantly lower summary scores for both mental and physical health over the study.
NP events in patients with SLE are of variable frequency, most commonly present early in the disease course and adversely impact patients' quality of life over time. Events attributed to non-SLE causes are more common than those due to SLE, although the latter have a more favourable outcome.
PurposeThe efficacy of antimalarials, especially hydroxychloroquine (HCQ), in preventing flares of systemic lupus erythematosus (SLE) is well demonstrated, but its effectiveness is impaired by ...non-adherence to treatment, reported to vary between 3% and 85%.We aimed to assess the associations of baseline severe non-adherence to HCQ, objectively assessed by HCQ serum levels, and risks of SLE flares, damage, and mortality over 5 years of follow-up.MethodsThe SLICC Inception Cohort is a multicentric prospective study of SLE patients from 31 centers in 11 countries. Patients were enrolled within 15 months of recognition of SLE (1997 ACR classification criteria). Serum HCQ levels of patients with HCQ prescription for at least 3 months, sampled at enrollment (or, if unavailable, during the first-year follow-up visits), were centrally measured. Severe non-adherence was defined by a serum HCQ level <106 ng/mL for a daily prescribed HCQ dose of 400 mg, and <53 ng/mL for a daily HCQ dose of 200 mg, respectively (1). SLE flare was defined by the occurrence of one of the following events within the first year following serum collection: (a) increase of at least four point in the SLEDAI-2K; (b) new start in prednisone (oral or pulse) or immunosuppressive agent; (c) a new renal involvement (active nephritis, nephrotic syndrome). Association between severe non-adherence and SLE flare was assessed with logistic regression models, further adjusted on potential confounders. Damage was assessed by the time until an increase ≥1 in the SLICC damage index (SDI), within the 5 years following HCQ measurement. Associations between severe non-adherence and either damage or mortality (from all cause) within 5 years following HCQ measurement were assessed with Cox proportional hazard models, adjusted on sex, education, and potential confounders.ResultsOf 1849 cohort subjects, 660 patients (88% women) were included. Median interquartile range serum HCQ level was 388 ng/mL (244–566) and 48 patients (7.3%) had severe HCQ non-adherence. No factors were clearly associated with severe non-adherence. A SLE flare occurred in 191 (28.9%) patients within the first year (28 58.3% non-adherent patients versus 163 26.6% other patients). In multivariate analysis, severe nonadherence was independently associated with the risk of flare (OR= 3.32; 95% CI 1.78–6.28).Within five years, 167 patients (25.3%) had ≥1 point increase of SDI. Severe on-adherence was associated with an increase in the SDI within each of the first 3 years (HR 1.92 at 3 years; 95% CI 1.05–3.50).Eleven patients died within 5 years, including 3 with severe non-adherence (unadjusted HR 5.41; 95% CI 1.43–20.39).Abstract S15.2 Figure 1Risk of damage, defined by a ≥1-point increase in SLICC ACR damage index, according to severe non-adherence to hydroxychloroquine (n=660)ConclusionsIn this large multicentric international prospective cohort, severe non-adherence was independently associated with the risk of SLE flare in the following year, with early damage, and 5-year mortality. As severe non-adherence is often unknown by the physician and since no predictive clinical or biological factors were identified, our results suggest the benefits of testing to detect severe non-adherence, to identify the patients at risk.
Diffuse idiopathic skeletal hyperostosis (DISH) is a common but little-studied disorder in the elderly that is infrequently recognized by physicians. Its prevalence in adults over 40 years of age is ...estimated at 3.8% for men and 2.6% for women. The present case-control study evaluated the history of pain and stiffness, radicular pain and enthesitis, physical findings on the musculoskeletal examination, and level of physical and psychologic disability in 130 persons: 56 patients with DISH, 43 control patients with spondylosis of the lumbar spine, and 31 healthy control patients. DISH patients were more likely to report a past history of upper extremity pain, medial epicondylitis of the elbow, enthesitis of the patella or heel, or dysphagia than spondylosis patients. They had more extremity and spinal stiffness and pain than healthy controls. DISH patients weighed more at a young age and their body mass index was greater at the time of the clinical evaluation than either spondylosis or healthy control patients. On musculoskeletal examination, DISH patients had a greater reduction in neck rotation and thoracic movements than either spondylosis patients or healthy controls, and had a greater reduction in lumbar movement than healthy controls. DISH patients had similar levels of spinal disability and physical disability overall, as measured by standardized indices, as spondylosis patients. No differences were found among the 3 groups of patients for the laboratory tests evaluated. DISH is clearly a distinct disorder with signs and symptoms that distinguish it from other causes of spinal complaint and from healthy individuals. It has the potential to cause major disability. Future studies need to address the natural history of DISH, pursue pathogenic mechanisms, and evaluate treatment modalities.
Neuropsychiatric events occur unpredictably in systemic lupus erythematosus (SLE) and most biomarker associations remain to be prospectively validated. This study examined a disease inception cohort ...of 1047 SLE patients to determine which autoantibodies at enrolment predicted subsequent neuropsychiatric events.
Patients with a recent SLE diagnosis were assessed prospectively for up to 10 years for neuropsychiatric events using the American College of Rheumatology case definitions. Decision rules of graded stringency determined whether neuropsychiatric events were attributable to SLE. Associations between the first neuropsychiatric event and baseline autoantibodies (lupus anticoagulant (LA), anticardiolipin, anti-β(2) glycoprotein-I, anti-ribosomal P and anti-NR2 glutamate receptor) were tested by Cox proportional hazards regression.
Disease duration at enrolment was 5.4 ± 4.2 months, follow-up was 3.6 ± 2.6 years. Patients were 89.1% female with mean (±SD) age 35.2 ± 13.7 years. 495/1047 (47.3%) developed one or more neuropsychiatric event (total 917 events). Neuropsychiatric events attributed to SLE were 15.4% (model A) and 28.2% (model B). At enrolment 21.9% of patients had LA, 13.4% anticardiolipin, 15.1% anti-β(2) glycoprotein-I, 9.2% anti-ribosomal P and 13.7% anti-NR2 antibodies. LA at baseline was associated with subsequent intracranial thrombosis (total n=22) attributed to SLE (model B) (HR 2.54, 95% CI 1.08 to 5.94). Anti-ribosomal P antibody was associated with subsequent psychosis (total n=14) attributed to SLE (model B) (HR 3.92, 95% CI 1.23 to 12.5, p=0.02). Other autoantibodies did not predict neuropsychiatric events.
In a prospective study of 1047 recently diagnosed SLE patients, LA and anti-ribosomal P antibodies are associated with an increased future risk of intracranial thrombosis and lupus psychosis, respectively.
Objective
To determine the frequency, clinical characteristics, associations, and outcomes of different types of peripheral nervous system (PNS) disease in a multiethnic/multiracial, prospective ...inception cohort of systemic lupus erythematosus (SLE) patients.
Methods
Patients were evaluated annually for 19 neuropsychiatric (NP) events including 7 types of PNS disease. SLE disease activity, organ damage, autoantibodies, and patient and physician assessment of outcome were measured. Time to event and linear regressions were used as appropriate.
Results
Of 1,827 SLE patients, 88.8% were female, and 48.8% were white. The mean ± SD age was 35.1 ± 13.3 years, disease duration at enrollment was 5.6 ± 4.2 months, and follow‐up was 7.6 ± 4.6 years. There were 161 PNS events in 139 (7.6%) of 1,827 patients. The predominant events were peripheral neuropathy (66 of 161 41.0%), mononeuropathy (44 of 161 27.3%), and cranial neuropathy (39 of 161 24.2%), and the majority were attributed to SLE. Multivariate Cox regressions suggested longer time to resolution in patients with a history of neuropathy, older age at SLE diagnosis, higher SLE Disease Activity Index 2000 scores, and for peripheral neuropathy versus other neuropathies. Neuropathy was associated with significantly lower Short Form 36 (SF‐36) physical and mental component summary scores versus no NP events. According to physician assessment, the majority of neuropathies resolved or improved over time, which was associated with improvements in SF‐36 summary scores for peripheral neuropathy and mononeuropathy.
Conclusion
PNS disease is an important component of total NPSLE and has a significant negative impact on health‐related quality of life. The outcome is favorable for most patients, but our findings indicate that several factors are associated with longer time to resolution.
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